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IPEX as a Result of Mutations in FOXP3.
- Source :
-
Clinical & Developmental Immunology . 2007, p1-5. 5p. 3 Diagrams. - Publication Year :
- 2007
-
Abstract
- Immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare disorder caused by mutations in the FOXP3 gene that result in the defective development of CD4+CD25+ regulatory T cells which constitute an important T cell subset involved in immune homeostasis and protection against autoimmunity. Their deficiency is the hallmark of IPEX and leads to severe autoimmune phenomena including autoimmune enteropathy, dermatitis, thyroiditis, and type 1 diabetes, frequently resulting in death within the first 2 years of life. Apart from its clinical implications, IPEX illustrates the importance of immunoregulatory cells such as CD4+CD25+ regulatory T cells. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 17402522
- Database :
- Academic Search Index
- Journal :
- Clinical & Developmental Immunology
- Publication Type :
- Academic Journal
- Accession number :
- 43934697
- Full Text :
- https://doi.org/10.1155/2007/89017